AUCTORES
Globalize your Research
Research Article | DOI: https://doi.org/10.31579/2693-2156/001
*Corresponding Author: Nasser Al Busaidi, Senior consultant pulmonologist at Royal Hospital, Muscat Sultanate of Oman.
Citation: Yasmeen Al Rawahi, Nasser Al Busaidi. Pirfenidone Experience in Idiopathic Pulmonary Fibrosis at a Tertiary Hospital in Oman. J Thoracic Disease and Cardiothoracic Surgery, 1(1); DOI: 10.31579/2693-2156/001
Copyright: © 2020 Nasser Al Busaidi. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Received: 31 January 2020 | Accepted: 12 February 2020 | Published: 17 February 2020
Keywords: idiopathic pulmonary fibrosis; anti-fibrotic drugs; perifendone; FVC; 6MWT; safety; adverse events
Objective: The aim of this study is to evaluate the clinical and safety profile of Pirfenidone treatment in IPF.
Methodology: This is a retrospective analysis of prospectively collected data via electronic system called “Al Shifa”. Data of all patients, who received Pirfenidone at the Royal Hospital in the period between April 2012 to April 2017, were retrieved into an excel sheet. The data retrieved at two time points: before starting the drug and last readings at time of the study.
Results: There were 90 patients with IPF that were treated with Pirfenidone, The age ranges between 35 to 91 years old. All patient assessed by Forced Vital Capacity (FVC) by spirometry, and 6-minutes’ walk test (6MWT). The mean percentage of pre- FVC (before starting the drug) was 59.3%, while follow-up FVC (the last reading at the time of entering the data) was 60.3%. The Pre-6MWT test; the mean of distance they manage to walk in 6 mints, was 339 meters, and follow-up-6MWT test was 320 meters, the difference between pre and post result in both tests are insignificant and hence these results indicated the stability of the disease. Also, only 16.6% experienced drug adverse effect; 10% due to gastro-intestinal symptoms, 2.2% weight loss and 4.4% due to Photo-sensitivity.
Conclusion: Pirfenidone stabilized IPF patients, as there was no decline in lung functions test and exercise capacity was preserved as demonstrated by the 6MWT. Perifenidone was well tolerated with acceptable safety profile.
Advances in Knowledge
- The first report on the clinical efficacy and safety of Pirfenidone for the treatment of patients with IPF in our region
- Middle aged group with equal gender representation of IPF
- Criteria of IPF was fulfilled by 80% of patients based on high-resolution CT scan and/or lung biopsy
Application to Patient Care
- Pirfenidone stabilized IPF patients, with preserved lung functions test and exercise capacity as demonstrated by the 6MWT
- Only 16.6% experienced drug adverse effect; 10% due to gastrointestinal symptoms, 2.2% weight loss and 4.4% due to Photo-sensitivity
- Education of patients and close follow-up is of paramount importance to improve the clinical outcomes.
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease, the disease carries high mortality and morbidity and if left untreated it may end up with respiratory failure and death within few years of diagnosis [1]. IPF characterized by inflammatory process in the interstitium, this process triggered by unknown factors, the inflammation in the interstitium then will be replaced by fibrosis [2]. Among all idiopathic interstitial pneumonias, IPF is the leading cause of chronic lung fibrosis which commonly ended up with terminal lung disease and respiratory failure [3]. IPF is worse than many cancers as the the median survival from the time of diagnosis is 3 years and in the US 40,000 patients die because of IPF every year [4].
Clinical trials such as Capacity (004 & 006) suggested that Pirfenidone which is an anti-fibrotic and anti-inflammatory drug reduces the deterioration of patients with IPF in lung function and exercise capacity [5]. these result also supported by the findings in ASCEND Clinical Trials, these trials showed that patients with IPF on Pirfenidone when compared to placebo group it reduces the disease progression as seen in lung function, exercise capacity and progression free survival, this study also showed good safety profile of Pirfenidone [6]. Because of these encouraging results, pirfenidone is approved for the treatment of IPF in many countries. We found no studies were done in Oman about the clinical and safety profile of the use of Pirfenidone for the treatment of IPF.
Is to measure the safety and efficacy of Pirfenidone.
To measure the safety and efficacy of Pirfenidone in our patients and reporting side effect of Pirfenidone.
The efficacy of Pirfenidone in patient with IPF as assessed by lungs function test and 6MWT.
This is a retrospective analysis of data obtained prospectively via an electronic health system called “Al Shifa”. All patients with IPF are evaluated at the Royal Hospital, which is a tertiary care hospital located at the capital and the health care is free for all citizens from all over the country. Pirfenidone is only available at the Royal Hospital. The study analyzes the data of all patients seen in the period between April 2012 to April 2017. The data retrieved at two time points: before starting the drug and last readings at time of the study. All patient assessed by Forced Vital Capacity (FVC) by spirometry, and 6-minutes’ walk test (6MWT). All patients that continued using the drug till the time of data collection were included and those who stopped using the drug for a less than a year were excluded from the analysis but reasons for stoppage of drug were evaluated.
The efficacy of the drug was assessed by Forced vital capacity (FVC); one parameter of lung function test; by comparing the result of FVC before starting Pirfenidone (Pre-FVC) with the last test done for the patient (Follow up-FVC), the patient also assessed by six mint walk test (6MWT) as Pre-6MWT (before start the drug) and Follow up-6MWT (done at the last visit).
The study was approved by Royal Hospital ethics committee
Data analysis
All the parameters collected from al shifa computer health system into and excel sheet and then transfer into SPSS statistical program. tTe data was further analyzed using specific statistical formulas (e.g chi square and student t –test) as indicated.
During the study period of five years, 90 adult patients with diagnosis of Interstitial Lung Diseases (ILD) were managed with Pirfenidone. Their age ranges between 35 -91-year-old with mean (SD) of 64 (15) years and 50% were females. (as seen in table 1)
The criteria of IPF was fulfilled by 80% of patients based on high resolution CT scan and/or lung biopsy. The remaining 20% were not IPF; but had Usual Interstitial Pneumonitis (UIP) picture in HRCT with underlying Connective Tissue disease (CTD).
The doses of the Pirfenidone ranged between 1800 to 2400 mg per day in 57.8% of the patients and the rest received sub-optimal dose of the drug ranging between 800 mg to 1600 mg per day. 50% of the patients continued using the drug till the time of data collection, the rest stopped using the drug over the 5 years but the all patients whom used the drug less than a year was excluded from the analysis, the patients stopped the drugs for variable reasons; 20% lost follow up, 14.4 % of the patient died during these 5 years, 7.8% due to adverse effect of the drug, 6.6% stopped by physician for no obvious reason and 1.2% was patients’ self-decision.
Over the 5 years period, 16.6% of patients experienced drug adverse effect; 10% due to gastro- intestinal (GI) side effects, 4.4% due to photo-sensitivity and 2.2% due to weight loss. however, 7.8% stopped the drug due to adverse effect, the rest was managed symptomatically or by reducing the dose of the drug. (as seen in table 1)
Figure 1 and as seen in table 1 showed the mean % of FVC before starting Pirfenidone (Pre-FVC) with the last test done for the patient (Follow up-FVC). The pre- FVC ranged between 29% predicted (the lowest) to 112% predicted (the highest) and the mean was 59.3% predicted, whereas Follow-FVC ranged between 27% predicted (the lowest) to 98% predicted (the highest) with the mean of 60.3% predicted.
Figure 2 and as seen in table 1 showed six mint walk test (6MWT) as Pre-6MWT (before the drug was started) and follow up-6MWT (done at the last visit). The Pre-6MWT distance walked during 6 mints ranged between 150 meters (the lowest) to 552 meters (the highest) with a mean of 339 meters, whereas the followup-6MWT walked by the patients during the test ranges between 90 meters (the lowest) to 573 meters (the highest) with mean of 320 meters.
The approval of Pirfenidone was based on data from a large, placebo-controlled Phase III study known as CAPACITY (studies 004 and 006) and is supported by other large Phase III trials known as ASCEND. In the ASCEND study, the primary endpoint is the percent changes in Forced Vital capacity (FVC), this study showed that’s more patients who received Pirfenidone had better lung function test at the end of the study as the drug delay the decline in FVC [6].
The present study is the first report on the clinical efficacy and safety of Pirfenidone for the treatment of patients with IPF in Oman. Pirfenidone stabilized IPF patients, as there was no decline in lung functions test and exercise capacity was preserved as demonstrated by the 6 mints walk test (6-MWT). Only 16.6% experienced drug adverse effect; 10% due to gastro-intestinal symptoms, 2.2% weight loss and 4.4% due to Photo-sensitivity.
Harari et.al reported that IPF effected people aged above 64 years and most of the patients were men (75%) than women (25%) [7]. Ryo et.al study showed comprised of 16 females and 60 males of 76 total number of patients diagnosis with IPF and the mean age of affected people with IPF is 70.5 years [8]. In our study, IPF patients age ranged between 35 -91-year-old with a mean of 64 and effected male and female equally.
Over the 5 years’ period, 16.6% experienced drug side effect; 10% due to gastro-intestinal (GI) side effects, 4.4% due to Photo-sensitivity and 2.2% due to weight loss. Hoffmann et.al study showed that most serious adverse events seen in people who received Pirfenidone when compared to those were in placebo: sensitivity to light or rash (9.0 percent vs. 1.0 percent) and gastrointestinal (GI) side effects that caused 2.2 percent of patients to stop the drug compared to only 1.0 percent of those who used placebo [6]. Anorexia as adverse effect was 42% as demonstrated by Ryo and his colleagues, this study also showed photosensitivity in 18.4%, fatigability 14.5% and GI adverse effect in 11.8% [8].
The pre- Force Vital capacity (pre-FVC) ranges between 29% predicted (the lowest) to 112% predicted (the highest) with mean of 59.3% predicted compared to Follow-FVC which ranges between 27% predicted (the lowest) to 98% predicted (the highest) with the mean of 60.3% predicted. Harari .S and his colleagues showed that the mean of FVC before starting Pirfenidone was 80%. One year later (follow-up test) with Pirfenidone treatment the mean FVC was 74% [7].
The Pre-6MWT test; the distance walked during 6 mints ranges between 150 meters to 552 meters with mean of 339 meters. In the follow up 6MWT test the distance walked by the patient during the test ranges between 90 meters to 573 meters with mean of 320 meters, similar result was seen in Harari .S et. al study whom demonstrated that the distance walked in 6-MWT was 452 met before before starting the Pirfenidone and a year later with use of Pirfenidone the mean distance walked in 6-MWT was 421met [7].
In our study the cohort of patient with IPF treated with Pirfenidone drug the period of 2012- 2017 have been stable as indicated by 6MWT (339met vs 320met) and FVC (59.3% vs 60.3%) pre and flow up respectively.
Clearly Auctoresonline and particularly Psychology and Mental Health Care Journal is dedicated to improving health care services for individuals and populations. The editorial boards' ability to efficiently recognize and share the global importance of health literacy with a variety of stakeholders. Auctoresonline publishing platform can be used to facilitate of optimal client-based services and should be added to health care professionals' repertoire of evidence-based health care resources.
Journal of Clinical Cardiology and Cardiovascular Intervention The submission and review process was adequate. However I think that the publication total value should have been enlightened in early fases. Thank you for all.
Journal of Women Health Care and Issues By the present mail, I want to say thank to you and tour colleagues for facilitating my published article. Specially thank you for the peer review process, support from the editorial office. I appreciate positively the quality of your journal.
Journal of Clinical Research and Reports I would be very delighted to submit my testimonial regarding the reviewer board and the editorial office. The reviewer board were accurate and helpful regarding any modifications for my manuscript. And the editorial office were very helpful and supportive in contacting and monitoring with any update and offering help. It was my pleasure to contribute with your promising Journal and I am looking forward for more collaboration.
We would like to thank the Journal of Thoracic Disease and Cardiothoracic Surgery because of the services they provided us for our articles. The peer-review process was done in a very excellent time manner, and the opinions of the reviewers helped us to improve our manuscript further. The editorial office had an outstanding correspondence with us and guided us in many ways. During a hard time of the pandemic that is affecting every one of us tremendously, the editorial office helped us make everything easier for publishing scientific work. Hope for a more scientific relationship with your Journal.
The peer-review process which consisted high quality queries on the paper. I did answer six reviewers’ questions and comments before the paper was accepted. The support from the editorial office is excellent.
Journal of Neuroscience and Neurological Surgery. I had the experience of publishing a research article recently. The whole process was simple from submission to publication. The reviewers made specific and valuable recommendations and corrections that improved the quality of my publication. I strongly recommend this Journal.
Dr. Katarzyna Byczkowska My testimonial covering: "The peer review process is quick and effective. The support from the editorial office is very professional and friendly. Quality of the Clinical Cardiology and Cardiovascular Interventions is scientific and publishes ground-breaking research on cardiology that is useful for other professionals in the field.
Thank you most sincerely, with regard to the support you have given in relation to the reviewing process and the processing of my article entitled "Large Cell Neuroendocrine Carcinoma of The Prostate Gland: A Review and Update" for publication in your esteemed Journal, Journal of Cancer Research and Cellular Therapeutics". The editorial team has been very supportive.
Testimony of Journal of Clinical Otorhinolaryngology: work with your Reviews has been a educational and constructive experience. The editorial office were very helpful and supportive. It was a pleasure to contribute to your Journal.
Dr. Bernard Terkimbi Utoo, I am happy to publish my scientific work in Journal of Women Health Care and Issues (JWHCI). The manuscript submission was seamless and peer review process was top notch. I was amazed that 4 reviewers worked on the manuscript which made it a highly technical, standard and excellent quality paper. I appreciate the format and consideration for the APC as well as the speed of publication. It is my pleasure to continue with this scientific relationship with the esteem JWHCI.
This is an acknowledgment for peer reviewers, editorial board of Journal of Clinical Research and Reports. They show a lot of consideration for us as publishers for our research article “Evaluation of the different factors associated with side effects of COVID-19 vaccination on medical students, Mutah university, Al-Karak, Jordan”, in a very professional and easy way. This journal is one of outstanding medical journal.
Dear Hao Jiang, to Journal of Nutrition and Food Processing We greatly appreciate the efficient, professional and rapid processing of our paper by your team. If there is anything else we should do, please do not hesitate to let us know. On behalf of my co-authors, we would like to express our great appreciation to editor and reviewers.
As an author who has recently published in the journal "Brain and Neurological Disorders". I am delighted to provide a testimonial on the peer review process, editorial office support, and the overall quality of the journal. The peer review process at Brain and Neurological Disorders is rigorous and meticulous, ensuring that only high-quality, evidence-based research is published. The reviewers are experts in their fields, and their comments and suggestions were constructive and helped improve the quality of my manuscript. The review process was timely and efficient, with clear communication from the editorial office at each stage. The support from the editorial office was exceptional throughout the entire process. The editorial staff was responsive, professional, and always willing to help. They provided valuable guidance on formatting, structure, and ethical considerations, making the submission process seamless. Moreover, they kept me informed about the status of my manuscript and provided timely updates, which made the process less stressful. The journal Brain and Neurological Disorders is of the highest quality, with a strong focus on publishing cutting-edge research in the field of neurology. The articles published in this journal are well-researched, rigorously peer-reviewed, and written by experts in the field. The journal maintains high standards, ensuring that readers are provided with the most up-to-date and reliable information on brain and neurological disorders. In conclusion, I had a wonderful experience publishing in Brain and Neurological Disorders. The peer review process was thorough, the editorial office provided exceptional support, and the journal's quality is second to none. I would highly recommend this journal to any researcher working in the field of neurology and brain disorders.
Dear Agrippa Hilda, Journal of Neuroscience and Neurological Surgery, Editorial Coordinator, I trust this message finds you well. I want to extend my appreciation for considering my article for publication in your esteemed journal. I am pleased to provide a testimonial regarding the peer review process and the support received from your editorial office. The peer review process for my paper was carried out in a highly professional and thorough manner. The feedback and comments provided by the authors were constructive and very useful in improving the quality of the manuscript. This rigorous assessment process undoubtedly contributes to the high standards maintained by your journal.
International Journal of Clinical Case Reports and Reviews. I strongly recommend to consider submitting your work to this high-quality journal. The support and availability of the Editorial staff is outstanding and the review process was both efficient and rigorous.
Thank you very much for publishing my Research Article titled “Comparing Treatment Outcome Of Allergic Rhinitis Patients After Using Fluticasone Nasal Spray And Nasal Douching" in the Journal of Clinical Otorhinolaryngology. As Medical Professionals we are immensely benefited from study of various informative Articles and Papers published in this high quality Journal. I look forward to enriching my knowledge by regular study of the Journal and contribute my future work in the field of ENT through the Journal for use by the medical fraternity. The support from the Editorial office was excellent and very prompt. I also welcome the comments received from the readers of my Research Article.
Dear Erica Kelsey, Editorial Coordinator of Cancer Research and Cellular Therapeutics Our team is very satisfied with the processing of our paper by your journal. That was fast, efficient, rigorous, but without unnecessary complications. We appreciated the very short time between the submission of the paper and its publication on line on your site.
I am very glad to say that the peer review process is very successful and fast and support from the Editorial Office. Therefore, I would like to continue our scientific relationship for a long time. And I especially thank you for your kindly attention towards my article. Have a good day!
"We recently published an article entitled “Influence of beta-Cyclodextrins upon the Degradation of Carbofuran Derivatives under Alkaline Conditions" in the Journal of “Pesticides and Biofertilizers” to show that the cyclodextrins protect the carbamates increasing their half-life time in the presence of basic conditions This will be very helpful to understand carbofuran behaviour in the analytical, agro-environmental and food areas. We greatly appreciated the interaction with the editor and the editorial team; we were particularly well accompanied during the course of the revision process, since all various steps towards publication were short and without delay".
I would like to express my gratitude towards you process of article review and submission. I found this to be very fair and expedient. Your follow up has been excellent. I have many publications in national and international journal and your process has been one of the best so far. Keep up the great work.
We are grateful for this opportunity to provide a glowing recommendation to the Journal of Psychiatry and Psychotherapy. We found that the editorial team were very supportive, helpful, kept us abreast of timelines and over all very professional in nature. The peer review process was rigorous, efficient and constructive that really enhanced our article submission. The experience with this journal remains one of our best ever and we look forward to providing future submissions in the near future.
I am very pleased to serve as EBM of the journal, I hope many years of my experience in stem cells can help the journal from one way or another. As we know, stem cells hold great potential for regenerative medicine, which are mostly used to promote the repair response of diseased, dysfunctional or injured tissue using stem cells or their derivatives. I think Stem Cell Research and Therapeutics International is a great platform to publish and share the understanding towards the biology and translational or clinical application of stem cells.
I would like to give my testimony in the support I have got by the peer review process and to support the editorial office where they were of asset to support young author like me to be encouraged to publish their work in your respected journal and globalize and share knowledge across the globe. I really give my great gratitude to your journal and the peer review including the editorial office.
I am delighted to publish our manuscript entitled "A Perspective on Cocaine Induced Stroke - Its Mechanisms and Management" in the Journal of Neuroscience and Neurological Surgery. The peer review process, support from the editorial office, and quality of the journal are excellent. The manuscripts published are of high quality and of excellent scientific value. I recommend this journal very much to colleagues.
Dr.Tania Muñoz, My experience as researcher and author of a review article in The Journal Clinical Cardiology and Interventions has been very enriching and stimulating. The editorial team is excellent, performs its work with absolute responsibility and delivery. They are proactive, dynamic and receptive to all proposals. Supporting at all times the vast universe of authors who choose them as an option for publication. The team of review specialists, members of the editorial board, are brilliant professionals, with remarkable performance in medical research and scientific methodology. Together they form a frontline team that consolidates the JCCI as a magnificent option for the publication and review of high-level medical articles and broad collective interest. I am honored to be able to share my review article and open to receive all your comments.
“The peer review process of JPMHC is quick and effective. Authors are benefited by good and professional reviewers with huge experience in the field of psychology and mental health. The support from the editorial office is very professional. People to contact to are friendly and happy to help and assist any query authors might have. Quality of the Journal is scientific and publishes ground-breaking research on mental health that is useful for other professionals in the field”.
Dear editorial department: On behalf of our team, I hereby certify the reliability and superiority of the International Journal of Clinical Case Reports and Reviews in the peer review process, editorial support, and journal quality. Firstly, the peer review process of the International Journal of Clinical Case Reports and Reviews is rigorous, fair, transparent, fast, and of high quality. The editorial department invites experts from relevant fields as anonymous reviewers to review all submitted manuscripts. These experts have rich academic backgrounds and experience, and can accurately evaluate the academic quality, originality, and suitability of manuscripts. The editorial department is committed to ensuring the rigor of the peer review process, while also making every effort to ensure a fast review cycle to meet the needs of authors and the academic community. Secondly, the editorial team of the International Journal of Clinical Case Reports and Reviews is composed of a group of senior scholars and professionals with rich experience and professional knowledge in related fields. The editorial department is committed to assisting authors in improving their manuscripts, ensuring their academic accuracy, clarity, and completeness. Editors actively collaborate with authors, providing useful suggestions and feedback to promote the improvement and development of the manuscript. We believe that the support of the editorial department is one of the key factors in ensuring the quality of the journal. Finally, the International Journal of Clinical Case Reports and Reviews is renowned for its high- quality articles and strict academic standards. The editorial department is committed to publishing innovative and academically valuable research results to promote the development and progress of related fields. The International Journal of Clinical Case Reports and Reviews is reasonably priced and ensures excellent service and quality ratio, allowing authors to obtain high-level academic publishing opportunities in an affordable manner. I hereby solemnly declare that the International Journal of Clinical Case Reports and Reviews has a high level of credibility and superiority in terms of peer review process, editorial support, reasonable fees, and journal quality. Sincerely, Rui Tao.
Clinical Cardiology and Cardiovascular Interventions I testity the covering of the peer review process, support from the editorial office, and quality of the journal.
